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Autoimmune Hypophysitis in Systemic Lupus Erythematosus: S F, S S P R R T C
Autoimmune Hypophysitis in Systemic Lupus Erythematosus: S F, S S P R R T C
Autoimmune Hypophysitis in Systemic Lupus Erythematosus: S F, S S P R R T C
Autoimmune Hypophysitis in Systemic Herein, we report a rare case of lupus in a male child who
presented with features of central hypothyroidism and diabetes
Lupus Erythematosus insipidus that was diagnosed as SLE-associated AH. He was
treated with pulse methylpre-dnisolone and cyclophosphamide
with hormone replacement.
A 14-year-old male child, fourth issue of a non-
Autoimmune hypophysitis (AH) is a rare autoimmune disease consanguineous marriage was admitted with history of fever,
that occurs when the pituitary gland is infiltrated with weight loss, pallor and generalized weakness since one month.
lymphocytes and plasma cells, leading to impaired hormonal There was no history of rash, bleeding manifestations,
secretion. Rare cases of association of systemic lupus erythe- abdominal distension, night sweats, oral ulcers, icterus,
matosus (SLE) with AH have been reported in literature but headaches, visual disturbances or joint swelling. He had received
mainly in adult population. AH commonly involves anterior multiple oral antibiotics with no improvement. In the past, he
pituitary; labelled as lymphocytic adenohypophysitis (LAH) had suffered a stroke at ten years of age with MRI brain showing
but it can also involve posterior pituitary which is called acute lacunar infarct in right corona radiata. Birth history was
lymphocytic infundibulo-neurohypophysitis (LINH) [1-4]. uneventful and he was immunized as per schedule.
Anthropometric parameters and vitals, including blood posterior pituitary bright spot on T1-weighted MRI. The gold
pressure, were normal. Clinical examination revealed malar rash, standard of diagnosis is pituitary biopsy which reveals massive
oral ulcers, severe pallor with moderate hepato-splenomegaly. infiltration of lymphocytes and plasma cells in the pituitary
Laboratory investigations revealed anemia (Hb, 6.4 g/dL), direct gland but is usually denied by patients due to its invasive nature.
Coombs test (DCT) positive, normal white blood cell (WBC) Our patient is similar to the case reported by Jing, et al. [4] in
counts (WBC, 7.9×109/L, Neutrophils 49%, Lymphocyctes which a 15 year lupus child revealed low levels of sex hormones,
51%), thrombocytopenia (Platelet count, 30,000/cmm), raised thyroid hormones and serum cortisol with MRI of pituitary
ESR (81 mm at end of one hour), prolonged activated partial region demonstrating an enlargement of the pituitary stalk. She
thromboplastin time (APTT) [Test, 54 sec (26.9-36.3)], high was diagnosed as LINH associated with SLE and responded
spot urine protein creatinine ratio (0.9, normal <0.2) with well to glucocorticoids and cyclophosphamide [4]. We treated
normal liver enzymes, serum electrolytes and X-Ray chest. our patient with steroids, cyclophosphamide, hydroxy-chloro-
Immunological investigations showed strongly positive anti- quine, hormone replacement and warfarin with significant
nuclear antibody (ANA titres 1:2560, speckled pattern), low clinical improvement in constitutional symptoms, normali-
serum complement C3 (C3-60 mg/dL; normal range 82-173 mg/ zation of acute phase reactants, complement levels, thyroid
dL) and C4 (C4 11.2 mg/dL; normal range: 13-46 mg/dL), function and urine proteinuria at 3 months of follow up.
positive anti-cardiolipin IgM antibody, beta-2 glycoprotein Warfarin was added to treatment protocol considering positive
IgM antibody and lupus anti-coagulant. Anti-double stranded antiphospholipid antibody and history of prior lacunar infarct.
DNA antibody and anti-Smith (anti-Sm) antibody were To the best of our knowledge this is the first case reported from
negative. Ophthalmology examination showed retinal India with features of AH in a case of juvenile SLE.
hemorrhages. Thyroid function test revealed central
Our case demonstrates association of SLE and AH in
hypothyroidism [low free T3 (<1 pg/mL), low free T4 (0.46 ng/
children. In the presence of central hypothyroidism and
dL), and low TSH (<0.01uIU/mL)], with positive anti-thyroid
diabetes insipidus in a lupus patient, endocrine hormonal
peroxidase (anti-TPO) antibodies. During the hospital stay,
evaluation and an MRI of pituitary gland is warranted to rule
child started developing delirium and agitation along with
out AH.
polyuria. Serum osmolality was high (320 mOsm/kg). Magnetic
resonance imaging (MRI) showed absence of posterior pituitary SUNIL KAPUR,1 JITENDRA OSWAL,1* RAHUL JAHAGIRDAR2
bright spot in T1-weighted imaging consistent with diabetes AND VIJAY VISWANATHAN1
insipidus. Serum cortisol, prolactin, and sex hormone levels 1Pediatric Rheumatology and 2Endocrinology,
were within normal limit. Parents did not consent for renal Department of Pediatrics,
biopsy. Although desirable, IgG4-related disease (IgG4-RD) Bharati Vidyapeeth University Medical College Hospital and
workup could not be done due to financial constraints. A Research Centre, Pune, Maharashtra, India.
diagnosis of SLE with multiple organ involvement with AH was *dmd.bharatihospital@gmail.com
made. He was initiated on intravenous pulse REFERENCES
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